European Journal of Radiology, 14 (1992) 0
1992 Elsevier
EURRAD
Science Publishers
201-203
B.V. All rights reserved. 0720-048X/92/$05.00
201
00244
Aggressive neuroblastoma with initial pulmonary metastases and kidney involvement simulating Wilms’ tumor M. Panuel’, ‘Department
B. Bourliere-Najean’,
of Pediatric Radiology, ‘Department
J.-C. Gentet2, C. Scheiner3, A. Delarue4, F. Faure’ and P. Devred’
of Pediatric Oncology, jDepartment
ofPathology.and
4Department of Pediatric Surgery. CHU
Timone, Marseille, France
(Received
13 June 1991; accepted
Key words: Neoplasm,
after revision 17 October
1991)
in infants and children; Neuroblastoma
Introduction Wilms’ tumor and neuroblastoma are the commonest retroperitoneal neoplasms in children. Neuroblastoma may invade the kidney and appear to be intrarenal [ 11. If pulmonary metastases occur in approximately 30% of cases of Wilms’ tumor, they are a very rare initial complication of neuroblastoma [2]. Case report A three-year-old female child was referred to the medical clinic for abdominal pain and distension. The patient had no significant past medical history. Physical examination revealed a huge abdominal mass, predominant in the right flank, pallor and weight loss. Plain radiography showed a mass in the right upper quadrant of the abdomen, without any calcification. Ultrasonography showed this mass, involving the right kidney and crossing the midline (Fig. 1). Only the lower pole of the right kidney was recognizable. The mass was heterogeneous and hyperechogenic compared to the liver parenchyma. The inferior vena cava was thrombosed and the thrombus involved the right atrium and was lying in the right ventricle during the diastol. There was an hepatic nodule, hypoechogenic compared to the liver parenchyma with an hyperechogenic rim. CT-scan showed the abdominal mass crossing midline, displacing the aorta to the left side, involving the right Address for reprints: Dr. M. Panuel, Department of Pediatric Radiology, CHU Timone, 13385 Marseille Cedex 05, France.
renal parenchyma and encasing the right renal vascular pedicle. The right renal vein was not clearly defined. This mass was heterogeneous before and during injection of contrast media, with poor contrast enhancement. The thrombus of the inferior vena cava and the right atrium was visible (Fig. 3A). The hepatic nodule appeared hypodense without contrast enhancement (Figs. 2A and 3B). Chest radiography revealed diffuse bilateral pulmonary nodules which were confirmed on CT-scan (Fig. 3B). On MRI coronal sections, the involvement of the heart is also clearly defined (Fig. 4). Serum and urinary catecholamine levels and neuron-
Fig. 1. Longitudinal sonogram of the right kidney shows the heterogeneous mass. The lower pole of the kidney is recognizable.
Fig. 2. (A) Contrast-enhanced CT scan shows the intrarenal appearance of the tumor, with a rim of renal parenchyma surrounding it. However, the tumor encases the renal vessels (arrow). (B) Contrast-enhanced CT scan shows the heterogeneous mass, with necrosis, displacing the aorta and a ‘bull-eye’ nodule in the left lobe of the liver. The inferior vena cava is not visible.
Fig. 3. (A) Sonogram of the heart: the thrombus involves the right atrium and lies into the right ventricle during the diastole. (B) Pulmonary CT scan: several nodules are visible.
specific enolase serum level were elevated and the diagnosis of neuroblastoma was made despite these radiological patterns simulating Wilms’ tumor. The histological studies confirmed the diagnosis with undifferentiated appearance. In spite of chemotherapy, the disease progressed, and the patient died 2 months later during surgery for acute tumor hemorrhage. Necropsy confirmed the diagnosis of right adrenal neuroblastoma invading the right kidney, neoplasic thrombus of the inferior vena cava, and hepatic and pulmonary involvement. Discussion An intrarenal mass in a child may occasionally be a neuroblastoma. Rosenfield et al. [l] reviewed six cases
Fig. 4. MR Tl-weighted (0.5 T; SE 400/26) coronal image: the thrombus is visible in the upper part of the inferior vena cava (arrow).
203
of aggressive neuroblastoma simulating Wilms’ tumor. The mean age of these children was 2 years and 4 months. Only one child had lung metastases. Five of these children died within 18 months after the onset of the disease, only one was survived but with bone metastases and poor response to treatment. The tumor, because of its particular aggressive nature, invades the kidney rather than displaces it. Oliphant et al. [3] suggest that the tumor infiltrates the sub-peritoneal space and spreads along the aorta or inferior vena cava and their branches, along the renal vessels in the renal pedicle. Thus, it directly invades the renal hilus. In our patient, the encasement of the renal artery and its branches was a good sign of neuroblastoma. Rather unusual was the thrombus of the inferior vena cava and right heart. This finding was recently reported by Day et al. [4] in three cases, but with only one histologically proven case. We could explain the inferior vena caval thrombus as a neoplastic thrombosis secondary to the renal involvement as seen in Wilms’ tumor. Histologic findings of our case and those of the literature [ 1,3] are those of a highly malignant neuroblastoma with small anaplastic cells and without rosette formation or ganglion differentiation. In one report [I], the tumor sample examined in order to determine the number of copies of the N-myc oncogene showed a marked genomit amplification, which seems to corroborate the aggressiveness of neuroblastoma and a poor prognosis [ 51; unfortunately, this study was not performed in our case.
Pulmonary metastases are not a common complication of neuroblastoma and are very rare in the initial tumor evaluation (3 cases out of 104 [2]). The pulmonary involvement is of three types : direct extension, hematogenous or lymphangitic spread. In our patient, the lung involvement was certainly of hematogeneous type because of inferior vena caval and right atria1 thrombosis. Pulmonary involvement seems to represent a widely disseminated metastatic disease and is a sign of very poor prognosis. To conclude, we would stress that abdominal neuroblastoma can be associated with renal involvement, inferior vena caval thrombus and initial pulmonary metastases. References Rosenfield NS, Leonidas JC, Barwick KW. Aggressive neuroblastoma simulating Wilms’ tumor. Radiology 1988; 166: 165-167. Towbin R, Gruppo RA. Pulmonary metastases in neuroblastoma. AJR 1982; 138: 75-78. Oliphant M, Berne AS. Mechanism ofdirect spread of abdominal neuroblastoma: CT demonstration and clinical implications. GastroIntest Radio1 1987; 12: 59-66. Day DL, Johnson R, Cohen MD. Abdominal neuroblastoma with inferior vena caval tumor thrombus: report of three cases (one with right atria1 extension). Pediatr Radio1 1991; 21: 205-207. Seeger RC, Brodeur GM, Sather H. Association of multiple copies of the N-myc oncogene with rapid progression of neuroblastoma. N Engl J Med 1985; 313: 111-l 16.
1992 Elsevier
EURRAD
Science Publishers
201-203
B.V. All rights reserved. 0720-048X/92/$05.00
201
00244
Aggressive neuroblastoma with initial pulmonary metastases and kidney involvement simulating Wilms’ tumor M. Panuel’, ‘Department
B. Bourliere-Najean’,
of Pediatric Radiology, ‘Department
J.-C. Gentet2, C. Scheiner3, A. Delarue4, F. Faure’ and P. Devred’
of Pediatric Oncology, jDepartment
ofPathology.and
4Department of Pediatric Surgery. CHU
Timone, Marseille, France
(Received
13 June 1991; accepted
Key words: Neoplasm,
after revision 17 October
1991)
in infants and children; Neuroblastoma
Introduction Wilms’ tumor and neuroblastoma are the commonest retroperitoneal neoplasms in children. Neuroblastoma may invade the kidney and appear to be intrarenal [ 11. If pulmonary metastases occur in approximately 30% of cases of Wilms’ tumor, they are a very rare initial complication of neuroblastoma [2]. Case report A three-year-old female child was referred to the medical clinic for abdominal pain and distension. The patient had no significant past medical history. Physical examination revealed a huge abdominal mass, predominant in the right flank, pallor and weight loss. Plain radiography showed a mass in the right upper quadrant of the abdomen, without any calcification. Ultrasonography showed this mass, involving the right kidney and crossing the midline (Fig. 1). Only the lower pole of the right kidney was recognizable. The mass was heterogeneous and hyperechogenic compared to the liver parenchyma. The inferior vena cava was thrombosed and the thrombus involved the right atrium and was lying in the right ventricle during the diastol. There was an hepatic nodule, hypoechogenic compared to the liver parenchyma with an hyperechogenic rim. CT-scan showed the abdominal mass crossing midline, displacing the aorta to the left side, involving the right Address for reprints: Dr. M. Panuel, Department of Pediatric Radiology, CHU Timone, 13385 Marseille Cedex 05, France.
renal parenchyma and encasing the right renal vascular pedicle. The right renal vein was not clearly defined. This mass was heterogeneous before and during injection of contrast media, with poor contrast enhancement. The thrombus of the inferior vena cava and the right atrium was visible (Fig. 3A). The hepatic nodule appeared hypodense without contrast enhancement (Figs. 2A and 3B). Chest radiography revealed diffuse bilateral pulmonary nodules which were confirmed on CT-scan (Fig. 3B). On MRI coronal sections, the involvement of the heart is also clearly defined (Fig. 4). Serum and urinary catecholamine levels and neuron-
Fig. 1. Longitudinal sonogram of the right kidney shows the heterogeneous mass. The lower pole of the kidney is recognizable.
Fig. 2. (A) Contrast-enhanced CT scan shows the intrarenal appearance of the tumor, with a rim of renal parenchyma surrounding it. However, the tumor encases the renal vessels (arrow). (B) Contrast-enhanced CT scan shows the heterogeneous mass, with necrosis, displacing the aorta and a ‘bull-eye’ nodule in the left lobe of the liver. The inferior vena cava is not visible.
Fig. 3. (A) Sonogram of the heart: the thrombus involves the right atrium and lies into the right ventricle during the diastole. (B) Pulmonary CT scan: several nodules are visible.
specific enolase serum level were elevated and the diagnosis of neuroblastoma was made despite these radiological patterns simulating Wilms’ tumor. The histological studies confirmed the diagnosis with undifferentiated appearance. In spite of chemotherapy, the disease progressed, and the patient died 2 months later during surgery for acute tumor hemorrhage. Necropsy confirmed the diagnosis of right adrenal neuroblastoma invading the right kidney, neoplasic thrombus of the inferior vena cava, and hepatic and pulmonary involvement. Discussion An intrarenal mass in a child may occasionally be a neuroblastoma. Rosenfield et al. [l] reviewed six cases
Fig. 4. MR Tl-weighted (0.5 T; SE 400/26) coronal image: the thrombus is visible in the upper part of the inferior vena cava (arrow).
203
of aggressive neuroblastoma simulating Wilms’ tumor. The mean age of these children was 2 years and 4 months. Only one child had lung metastases. Five of these children died within 18 months after the onset of the disease, only one was survived but with bone metastases and poor response to treatment. The tumor, because of its particular aggressive nature, invades the kidney rather than displaces it. Oliphant et al. [3] suggest that the tumor infiltrates the sub-peritoneal space and spreads along the aorta or inferior vena cava and their branches, along the renal vessels in the renal pedicle. Thus, it directly invades the renal hilus. In our patient, the encasement of the renal artery and its branches was a good sign of neuroblastoma. Rather unusual was the thrombus of the inferior vena cava and right heart. This finding was recently reported by Day et al. [4] in three cases, but with only one histologically proven case. We could explain the inferior vena caval thrombus as a neoplastic thrombosis secondary to the renal involvement as seen in Wilms’ tumor. Histologic findings of our case and those of the literature [ 1,3] are those of a highly malignant neuroblastoma with small anaplastic cells and without rosette formation or ganglion differentiation. In one report [I], the tumor sample examined in order to determine the number of copies of the N-myc oncogene showed a marked genomit amplification, which seems to corroborate the aggressiveness of neuroblastoma and a poor prognosis [ 51; unfortunately, this study was not performed in our case.
Pulmonary metastases are not a common complication of neuroblastoma and are very rare in the initial tumor evaluation (3 cases out of 104 [2]). The pulmonary involvement is of three types : direct extension, hematogenous or lymphangitic spread. In our patient, the lung involvement was certainly of hematogeneous type because of inferior vena caval and right atria1 thrombosis. Pulmonary involvement seems to represent a widely disseminated metastatic disease and is a sign of very poor prognosis. To conclude, we would stress that abdominal neuroblastoma can be associated with renal involvement, inferior vena caval thrombus and initial pulmonary metastases. References Rosenfield NS, Leonidas JC, Barwick KW. Aggressive neuroblastoma simulating Wilms’ tumor. Radiology 1988; 166: 165-167. Towbin R, Gruppo RA. Pulmonary metastases in neuroblastoma. AJR 1982; 138: 75-78. Oliphant M, Berne AS. Mechanism ofdirect spread of abdominal neuroblastoma: CT demonstration and clinical implications. GastroIntest Radio1 1987; 12: 59-66. Day DL, Johnson R, Cohen MD. Abdominal neuroblastoma with inferior vena caval tumor thrombus: report of three cases (one with right atria1 extension). Pediatr Radio1 1991; 21: 205-207. Seeger RC, Brodeur GM, Sather H. Association of multiple copies of the N-myc oncogene with rapid progression of neuroblastoma. N Engl J Med 1985; 313: 111-l 16.
